It's All I Can Do

About Cystic Fibrosis

Cystic fibrosis (CF) is a genetic disease in which excess mucus clogs the lungs, prevents food from being digested, and damages the reproductive system. It is the most common life-threatening genetically inherited disease affecting Caucasians.

In the bodies of people with CF, abnormal CFTR proteins are produced which changes the way chloride (salt) ions move in and out of cells. This affects the balance between salt and water in the body, causing the mucus that lines the lungs, pancreas, and other organs to become thicker and stickier. The fewer fully functioning CFTR protiens a person has, the worse the symptoms of the disease are.

CF cannot be “caught” like a cold or the flu. It’s genetic, and is caused by inheriting two copies of  an abnormal CFTR gene, one mutant gene from each parent. People who have one defective gene and one normal copy of the gene are said to be “carriers” of the CF gene. Carriers don’t usually have symptoms of CF, but they have fewer functioning CFTR protiens, and can pass on the mutated CFTR gene to their children.

In the lungs, the mucus plugs  airways leading to wheezing, coughing, and trouble breathing. This also puts those with CF at risk for lung and sinus infections. Bacteria can always be found in the airways of people with CF. Some of these bacteria can speed up the process of damage to the lungs.

As a person ages, the symptoms of CF become progressively worse, and often a double lung transplant is needed. Other complications arise, such as diabetes and and liver damage. On average, people with CF in North America live to the age of 35.

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